Immune-mediated rippling muscle disease and myasthenia gravis

2016 ◽  
Vol 299 ◽  
pp. 59-61 ◽  
Author(s):  
Mariela Bettini ◽  
Hernan Gonorazky ◽  
Marcelo Chaves ◽  
Ernesto Fulgenzi ◽  
Alejandra Figueredo ◽  
...  
2009 ◽  
Vol 19 (3) ◽  
pp. 223-228 ◽  
Author(s):  
Benedikt Schoser ◽  
Saiju Jacob ◽  
David Hilton-Jones ◽  
Wolfgang Müller-Felber ◽  
Christian Kubisch ◽  
...  

2006 ◽  
Vol 16 (9-10) ◽  
pp. 647 ◽  
Author(s):  
B.G.H. Schoser ◽  
D. Hilton-Jones ◽  
W. Müller-Felber ◽  
C. Kubisch ◽  
A. Vincent ◽  
...  

2021 ◽  
Vol 11 (8) ◽  
pp. 1035
Author(s):  
Maria Pia Giannoccaro ◽  
Patrizia Avoni ◽  
Rocco Liguori

The neuromuscular junction (NMJ) is the target of a variety of immune-mediated disorders, usually classified as presynaptic and postsynaptic, according to the site of the antigenic target and consequently of the neuromuscular transmission alteration. Although less common than the classical autoimmune postsynaptic myasthenia gravis, presynaptic disorders are important to recognize due to the frequent association with cancer. Lambert Eaton myasthenic syndrome is due to a presynaptic failure to release acetylcholine, caused by antibodies to the presynaptic voltage-gated calcium channels. Acquired neuromyotonia is a condition characterized by nerve hyperexcitability often due to the presence of antibodies against proteins associated with voltage-gated potassium channels. This review will focus on the recent developments in the autoimmune presynaptic disorders of the NMJ.


2021 ◽  
Vol 12 ◽  
Author(s):  
Farzad Fatehi ◽  
Kamyar Moradi ◽  
Ali Asghar Okhovat ◽  
Ghazaleh Shojatalab ◽  
Behnaz Sedighi ◽  
...  

Objectives: Myasthenia gravis (MG) is an immune-mediated neuromuscular disorder responsive to immunomodulatory treatments. 10–20% of MGs are not responsive to conventional first-line therapies. Here, we sought to investigate the efficacy and safety of rituximab therapy in the treatment of patients with refractory MG.Methods: In a 48-week, multicenter, open-labeled, prospective cohort setting, 34 participants with refractory MG were assigned to receive infusions of Zytux, which is a rituximab biosimilar, according to a validated protocol. Clinical, functional, and quality of life (QoL) measurements were recorded at baseline, and seven further visits using the Myasthenia Gravis Foundation of America (MGFA), Myasthenia Gravis Composite (MGC), Myasthenia Gravis Activities of Daily Living profile (MG-ADL), and Myasthenia Gravis Quality of Life (MGQoL-15) scales. Besides, the post-infusion side effects were systematically assessed throughout the study.Results: The correlation analysis performed by generalized estimating equations analysis represented a significant reduction of MGC, MG-ADL, and MGQoL-15 scores across the trial period. The subgroup analysis based on the patients' clinical status indicated a significant effect for the interaction between time and MGFA subtypes on MG-ADL score, MGC score, and pyridostigmine prednisolone dose, reflecting that the worse clinical condition was associated with a better response to rituximab. Finally, no serious adverse event was documented.Conclusions: Rituximab therapy could improve clinical, functional, and QoL in patients with refractory MG in a safe setting. Further investigations with larger sample size and a more extended follow-up period are warranted to confirm this finding.Clinical Trial Registration: The study was registered by the Iranian Registry of Clinical Trials (IRCT) (Code No: IRCT20150303021315N18).


2021 ◽  
Vol 12 ◽  
pp. 215013272110519
Author(s):  
Augustine Chavez ◽  
Charlotte Pougnier

An 82-year-old man presented with intermittent episodes of slurred speech during his evening meals after receiving the BNT162b2 COVID-19 vaccine. Thorough evaluation was conducted including lab work and EMG confirming a new diagnosis of late-onset myasthenia gravis. Despite treatment, the patient progressed rapidly to severe exacerbation requiring intubation and placement of a PEG tube. Infections provoking new diagnosis and exacerbations of myasthenia gravis have been reported. New diagnosis of myasthenia gravis associated with the COVID-19 vaccine is rarely reported. This case highlights the need for clinicians to be aware of the uncommon presenting symptoms in late-onset myasthenia gravis and the possibility of vaccine provoked diagnoses of immune mediated diseases.


2012 ◽  
Vol 53 (11) ◽  
pp. 661-663 ◽  
Author(s):  
E. T. Bell ◽  
C. S. Mansfield ◽  
F. E. James

2020 ◽  
Vol 62 (5) ◽  
pp. 579-592 ◽  
Author(s):  
Ali Aamer Habib ◽  
Ghazaleh Ahmadi Jazi ◽  
Tahseen Mozaffar

2012 ◽  
Vol 123 (6) ◽  
pp. e21-e22
Author(s):  
T. Liewluck ◽  
B. Goodman ◽  
M. Milone

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